Managing paediatric bleeding disorder patients undergoing surgical procedures: Leveraging the electronic medical record.

INTRODUCTION: Individuals with bleeding disorders have a high risk of bleeding complications with surgical procedures. Careful planning and management of peri-operative treatment is vital for their safety. Yet, inter-provider communication and communication between patients/families and providers is not reliable. AIM: Our haemophilia treatment centre (HTC) created a care gap report that used the electronic medical record to inform our team when patients with bleeding disorders were scheduled for procedures. METHODS: An electronic medical record-based patient registry was linked to the hospital's surgical schedule and a report was run daily by HTC staff for the upcoming 14 days. We determined the number of surgeries scheduled for patients with a bleeding disorder without the knowledge of the HTC, identified by the care gap report during the 6 months prior to and 2 years after implementing the report. RESULTS: Had the report been in effect 6 months prior, the majority of surgery cases would have been detected and planned for an average of 10 days prior to the procedure. Following implementation, the report identified 62 of 225 surgeries on patients with known bleeding disorders where the HTC did not have prior communication from the patient/family or surgical team. CONCLUSION: This surgery care gap report provides the date and time of procedures on bleeding disorder patients without relying on contact from patients/families or the surgical team. Its use has resulted in an improved peri-operative process for patients with bleeding disorders undergoing surgical procedures and potentially prevented surgery cancellations.

Ultrasound-guided left brachiocephalic vein cannulation in children with underlying bleeding disorders: a retrospective analysis.

OBJECTIVES: To evaluate the safety and effectiveness of ultrasound-guided left brachiocephalic vein cannulation in infants and children with underlying bleeding conditions. DESIGN: Retrospective cohort. SETTING: PICU of a tertiary pediatric hospital. PATIENTS: Thirty-four patients requiring central venous catheterization from January 2011 to January 2012. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Two pediatric intensivists, experienced in ultrasound-guided vessel cannulation, performed the ultrasound catheterization of the left brachiocephalic vein. Ultrasound equipment consisted of a standard ultrasound monitor with a linear 6-13 MHz probe. The ultrasound monitor was set on a resolution with a depth of 1.8 cm for infants and 2.2 cm for children. The "in-plane" technique was used for all patients. Thirty-four catheterizations were performed. Patient median age was 12.5 months (5.75-63.5 mo) and median weight was 9.25 kg (7-16.25 kg). The population of infants and children analyzed was composed of 25 patients with hematologic disorder (73%) treated with hematopoietic stem cell transplantation, five patients (15%) supported with extracorporeal membrane oxygenation for viral pneumonias, and four patients (12%) with uremic hemolytic syndrome. A 4F catheter was used in 79% of cases. Left brachiocephalic vein cannulation was successful in all 34 patients. Median time needed for cannulation was 350 seconds (277.5-450 s). The overall complication rate was 9% (3 of 34) and consisted of difficulty in advancing the guidewire after having pierced the vein. The time required for catheter positioning and complications was not associated with both lower body weight and body surface area of the patients (p > 0.05). Mean central venous catheter duration was 32 ± 4 days. CONCLUSIONS: Data reported in this retrospective study confirm the safety and effectiveness of ultrasound-guided left brachiocephalic vein catheterization in infants and children with underlying bleeding disorders.

[Advances in the pathogenesis and treatment of neurosurgical emergency with coagulation disorders].

Neurosurgical emergencies including intracranial hemorrhage and head trauma have high mortality and morbidity rates and meanwhile are often accompanied with coagulation disorders. On one hand, coagulation disorder follows traumatic brain injury;on the other hand, the increasing use of anticoagulant and antiplatelet treatment for cardiovascular diseases increases the risk of death among patients with brain trauma or bleeding. Once the intracranial pressure increases, such patients need emergency surgical intervention, but coagulation disorder is a relative contraindication. This article reviews the pathogenesis and treatment of coagulation disorders in patients with neurosurgical emergency. It also analyzes clinical monitoring indices for such patients and their variations and summarizes the strategies and measures of perioperative management.

[Trauma-associated bleeding in the severely injured. Relevance, risk stratification and current therapy approaches]. / Traumaassoziierte Blutung beim Schwerverletzten. Relevanz, Risikostratifizierung und aktuelle Therapieansätze.

Of all trauma-related deaths 40% are due to exsanguination. The causes for acute, hemorrhaging are uncontrolled bleeding sources and the development of acute posttraumatic coagulopathy. Clinical observations and recent research results emphasize the key role of this disorder in acute trauma care. The present synopsis summarizes the results from different analyses based on datasets from severely injured patients derived from the Trauma Register of the German Trauma Society (DGU) on frequency, potential triggers and strategies to manage acute posttraumatic coagulopathy. In an extension to this work a clinical scoring system for early identification of patients at high risk for ongoing bleeding is presented. High risk patients seem to benefit from a more balanced transfusion regimen.

[Haemostasis disorders and ways of its correction in HIV-positive pregnant women receiving chemopreventive antiretroviral therapy].

The analysis of 162 surgical deliveries cases in HIV-positive patients with antiretroviral therapy--induced haemostasis disturbances and thrombocytopenia was carried out. The article gives the perioperative management during surgical delivery.

Comparison of the rates of joint arthroplasty in patients with severe factor VIII and IX deficiency: an index of different clinical severity of the 2 coagulation disorders.

Data from the Italian Hemophilia Centres were collected to perform a retrospective survey of joint arthroplasty in patients with severe hemophilia. Twenty-nine of 49 hemophilia centers reported that 328 of the 347 operations were carried out in 253 patients with severe hemophilia A (HA) and 19 in 15 patients with severe hemophilia B (HB). When results were normalized to the whole Italian hemophilia population (1770 severe HA and 319 severe HB), patients with HA had a 3-fold higher risk of undergoing joint arthroplasty (odds ratio [OR], 3.38; 95% confidence interval [CI], 1.97-5.77; P < .001). These results were confirmed after adjustment for age, HIV, hepatitis C virus (HCV), and inhibitor in a Cox regression model (HR, 2.65; 95% CI, 1.62-4.33; P < .001). The survival analysis of time to joint arthroplasty in the subset of patients with severe HA was not affected by the severity of factor VIII (FVIII) gene mutations. A systematic review of literature articles reporting joint arthroplasties in HA and HB showed that the proportion of HA patients who had undergone arthroplasties was higher than that of HB patients, in agreement with the findings in our Italian cohort. These data suggest that the 2 inherited coagulation disorders have a different severity of clinical phenotype.

Endoscopic intra-hematomal evacuation of intracerebral hematomas - a suitable technique for patients with coagulopathies.

OBJECTIVES: To describe an endoscopic technique to evacuate acute intracerebral hemorrhage (ICH) using the balanced suction-irrigation method in patients with intrinsic or iatrogenic coagulopathies. METHODS: We report on our early experience with four patients with atypical ICH related to intrinsic and iatrogenic coagulopathies. In all patients, an endoscopic hematoma evacuation was performed using a navigated burrhole approach. The entry site and trajectory were planned according to the long axis of the hematoma. RESULTS: Every operation was carried out with the aid of neuronavigation. Gross total removal of the hematoma was not intended as first line, especially if eloquent areas could be avoided. Intra-hematomal evacuation leaving minimal hematoma remnants was performed in three of four patients. We report hematoma removal rates of approximately 90%. In all patients, a significant hematoma reduction was achieved, although residues were tolerated to limit neurological damage. No re-hemorrhage was observed. CONCLUSION: The endoscopic technique with the aid of neuronavigation may be an appropriate method to safely evacuate ICH in the acute stage in patients with intrinsic or iatrogenic coagulopathies.

Coagulation issues in vascular anomalies.

Vascular anomalies, comprised of vascular tumors and malformations, are frequently associated with coagulopathy. Recognition of and familiarity with these vascular anomaly-associated hematologic abnormalities prior to surgery or interventional procedures is essential for pre-operative pre-operative planning. Complicated coagulopathies present within the framework of either Kasabach-Merritt phenomenon (KMP) or localized intravascular coagulopathy (LIC), and their management benefits from the expertise of a hematologist for optimal intra- and peri­operative care. Furthermore, with the recent broadening of understanding of vascular anomalies and the addition of new classification sub-groups, distinctions of these two classic coagulopathy phenotypes have been recognized. This review summarizes the main features of these coagulopathies, described according to their vascular anomaly type, highlighting clinical aspects relevant to surgical management.

Rehabilitative of COVID-19 patients with acute lower extremity Ischemia and amputation.

OBJECTIVE: To evaluate the clinical characteristics and rehabilitation management of patients who undergo amputation for COVID-19-associated coagulopathy. METHODS: Clinical and laboratory data for 3 patients were analysed and their rehabilitative management discussed. RESULTS: The medical records of 3 patients who had undergone amputation due to acute lower extremity ischaemia and who were provided with rehabilitation in our COVID-19 unit were reviewed. CONCLUSION: Coagulation changes related to SARS-CoV-2 may complicate recovery from this devastating disease. The rehabilitation management of amputated patients for COVID-19 acute lower extremity ischaemia is based on a multilevel approach for clinical, functional, nutritional and neuropsychological needs. Based on this limited experience, a dedicated programme for this specific group of patients seems advantageous to warrant the best functional outcome and quality of life.

Contact factor deficiencies and cardiopulmonary bypass surgery: detection of the defect and monitoring of heparin.

Contact factor pathway deficiencies do not cause surgical bleeding but make heparin monitoring by the activated partial thromboplastin time (APTT) and activated clotting time (ACT) unreliable. Heparin monitoring during cardiopulmonary bypass (CPB) surgery in these patients is particularly challenging. Here we describe heparin monitoring during CPB using the chromogenic anti Xa assay in two patients with severe factor XII deficiency (FXII < 0.01 U/mL) and one patient with severe prekallikrein (PK) deficiency (PK < 0.01 U/mL). Anti Xa levels of the three patients during CPB varied between 3.8 and 4.8 U/mL in keeping with a control group (mean anti Xa 4.5 U/mL and ACT > 480 s). There were no bleeding or thrombotic complications. We also found that detection of severe PK deficiency by the APTT in the PK deficient patient was dependent on the reagent used and discuss the sensitivity of different APTT reagents for contact factor deficiencies. We conclude that the sensitivity of APTT methods for contact pathway deficiencies is highly variable and although insensitivity is not a clinical problem in terms of bleeding, it can be a cause of discrepancy between different APTT reagents and the ACT. This can lead to confusion about a possible haemorrhagic tendency and delays in surgery. If these patients need to undergo cardiac surgery requiring high dose heparin treatment, monitoring by chromogenic anti Xa assay is a good alternative.

Use of gelatin hemostatic matrices in patients with intraparenchymal hemorrhage and drug-induced coagulopathy.

In the routine practice of neurosurgery, the attainment of appropriate hemostasis during and after surgery is of the utmost importance. In the last few years, we have noticed that in several cases the standard coagulation methods (bipolar, Tabotamp, Spongostan) were not sufficient; in particular, patients with intraparenchymal hemorrhage under anticoagulant or antiplatelet therapy were observed to be the most difficult hemostasis cases, and thus those most frequently subjected to gelatin hemostatic matrices. We report our trial on 57 patients under anticoagulant or antiplatelet therapy and with intraparenchymal hemorrhage in which gelatin hemostatic matrices were used. The excellent results both in terms of outcome and decreased bleeding allow for regarding such a practice as safe and reproducible in these cases.

Bariatric Surgery Outcomes in Patients on Preoperative Therapeutic Anticoagulation: an Analysis of the 2015 to 2017 MBSAQIP.

BACKGROUND: Obesity has been found to be an independent predictor of adverse cardiac and pulmonary embolic events. As the popularity of bariatric surgery grows, surgeons are encountering more patients taking therapeutic anticoagulation medications preoperatively. This study aims to assess the safety of bariatric surgery on these patients. METHODS: Data was extracted from 2015 to 2017 using the MBSAQIP database. Included patients were those who underwent a primary LSG or LRYGB. A multivariable regression analysis was performed looking at 30-day outcomes for pre-operatively anticoagulated patients. A secondary propensity-matched analysis was performed comparing outcomes among patients undergoing LSG vs LRYGB. RESULTS: A total of 430,396 patients were analyzed, 11,013 (2.56%) of which were taking anticoagulation medications pre-operatively. Absolute 30-day complication rates (8.73% vs 3.36%, p < 0.001), bleed rates (3.78% vs 0.88%, p < 0.001), leak rates (0.55% vs 0.41%, p = 0.021), cardiac event rates (0.43% vs 0.06%, p < 0.001), and venous thromboembolism rates (0.68% vs 0.25%, p < 0.001) were significantly higher among pre-operatively anticoagulated patients. On multivariable analysis, pre-operative anticoagulation was found to be an independent predictor of postoperative bleeding (OR 2.76, CI 2.43-3.14, p < 0.001) and mortality (OR 2.08, CI 1.49-2.90, p < 0.001). The LRYGB was associated with a significantly higher complication rate compared to the LSG (13.27% vs 7.40%, p < 0.001) in the propensity-matched cohorts. CONCLUSIONS: Patients undergoing bariatric surgery on anticoagulation medications pre-operatively are at a significantly higher risk of adverse outcomes post-operatively. Patients who require long-term anticoagulation should undergo careful consideration before proceeding with bariatric surgery.

[Renunciation of routine coagulation diagnostics before ENT operations in children. Results of a survey into the implementation of this recommendation in Dresden]. / Verzicht auf eine Routinegerinnungsdiagnostik vor HNO-Eingriffen bei Kindern. Ergebnisse einer Umfrage zur Umsetzung dieser Empfehlung in Dresden.

UNLABELLED: In Germany a recommendation was introduced by experts from several medical associations concerning the renunciation of preoperative coagulation diagnostics in ENT interventions in children with inconspicuous bleeding history and published in July 2006. In August 2007 a survey concerning the implementation of this recommendation was sent to all pediatricians and ENT doctors working in medical offices in Dresden. RESULT: The survey was answered by 23 (49%) paediatricians out of 47 who were contacted and 8 (33%) out of 24 ENT doctors. Fifteen pediatricians (65%) and 3 ENT doctors (38%) have implemented the recommendation consequently and 6 respectively 3 occasionally. Only 2 pediatricians and 2 ENT doctors did not accept the recommendation. Four paediatricians and 4 ENT doctors expressed their concerns with the implementation of recommendation. Since the implementation of this recommendation 3 children suffered from bleeding complications in ambulant ENT operations but in no case a coagulation disorder was present. CONCLUSION: The implementation of the recommendation at the regional level is practicable. Its acceptance is obviously higher in paediatricians than in ENT doctors.

Small intestinal bacterial overgrowth (SIBO) and vitamin K-responsive coagulopathy: a previously unrecorded association.

A 17-year-old woman, with a history of three operations on the upper gut in early life and intermittent diarrhoea, presented with a history of epistaxis and leg ecchymosis for the previous 3 months. Initial investigation revealed mild anaemia, low serum albumin, moderately elevated aminotransferases and an exceedingly prolonged prothrombin time (PT) which was promptly shortened to normal by intravenous vitamin K. Additional investigations revealed a grossly abnormal glucose hydrogen breath test, a dilated duodenum and deficiencies of vitamins A, D and E. Repeated courses of antimicrobial agents caused prompt but transient shortening of PT and eventually a duodenal-jejunal anastomosis was performed. Since then, up to 36 months later, the patient has been in good general health and PT has been consistently normal with no vitamin K supplementation. Small intestinal bacterial overgrowth has previously been associated with several conditions but this is the first description of its association with vitamin K-responsive coagulopathy.

Risk Factors for Perioperative Blood Transfusions in Adult Spinal Deformity Surgery.

OBJECTIVE: Adult spinal deformity (ASD) surgery is associated with a high rate of perioperative blood transfusions, and it is important to understand the risk factors for perioperative blood transfusions to implement strategies to reduce transfusions. The aim of this study was to identify independent risk factors of perioperative blood transfusions in patients undergoing surgery for ASD. METHODS: A retrospective cohort study was performed using the American College of Surgeons National Surgical Quality Improvement Program database from 2010 to 2014. Adult patients undergoing surgery for ASD were separated into 2 cohorts based on whether they received a perioperative blood transfusion. Univariate and multivariate regression models were used to identify risk factors for blood transfusion. RESULTS: In our cohort of 5805 patients, 27.1% received a blood transfusion. Multivariate regression analysis showed that patient-specific risk factors were age 65 years or older (odds ratio [OR], 1.74; 95% confidence interval [CI], 1.49-2.03; P < 0.001), American Society of Anesthesiologists classification of 3 or greater (OR, 1.18; 95% CI, 1.01-1.37; P = 0.033), cardiac comorbidity (OR, 1.21; 95% CI, 1.03-1.41; P = 0.018) and bleeding disorder (OR, 2.01; 95% CI, 1.10-3.66; P = 0.023). Surgery-specific risk factors were a posterior approach (OR, 4.25; 95% CI, 3.46-5.22; P < 0.001), pelvic fixation (OR, 1.73; 95% CI, 1.36-2.20; P < 0.001), and osteotomy (OR, 2.08; 95% CI, 1.71-2.51; P < 0.001). Longer operative time was also a risk factor with a duration-dependent effect on the odds of blood transfusion. CONCLUSIONS: Recognition of patient- and surgery-specific risk factors for perioperative blood transfusion is important to identify patients who are at high risk and to implement strategies to minimize intraoperative blood loss and decrease healthcare costs.

Recombinant Activated Factor VII (Eptacog Alfa Activated, NovoSeven®) in Patients with Rare Congenital Bleeding Disorders. A Systematic Review on its Use in Surgical Procedures.

In the absence of definite guidelines in the area, we have carried a systemic review to provide a thorough overview concerning the efficacy and safety of recombinant activated factor VII (rFVIIa, NovoSeven®, Novo Nordisk A/S, Bagsværd, Denmark) in patients with Glanzmann's thrombasthenia (GT) and FVII deficiency, undergoing surgical procedures. PubMed, Web of Science, Scopus and EMBASE databases was employed for the search. Three multicenter registries were identified: the Glanzmann's Thrombasthenia Registry (GTR), the Seven Treatment Evaluation Registry (STER), and a German post-marketing surveillance registry (the WIRK study). In addition, data from 10 case-series and/or single-center experiences have been summarized. We have found that the following; perioperatively, the hemostatic effectiveness of rFVIIa was high in GT patients and in those with FVII deficiency undergoing both minor and major surgical procedures. Moreover, in all studies, rFVIIa was well tolerated. Thus, the current evidence shows an optimal perioperative safety/efficacy profile of rFVIIa in the setting of these rare bleeding disorders, and provides the rationale for further studies aimed at evaluating the optimal perioperative anti-hemorrhagic prophylaxis with rFVIIa in GT and in FVII deficient patients.

Practical Use of Thromboelastometry in the Management of Perioperative Coagulopathy and Bleeding.

Achieving hemostasis after complex cardiovascular and transplant surgical procedures is one of the greatest challenges anesthesiologists face. Preoperative coagulation disturbances due to underlying disease or antithrombotic therapy are common, and they are worsened by intraoperative blood loss and fluid replacement. The coagulation reactions in vivo are incredibly complex interactions among blood cells, proteins, and vasculature, standing in sharp contrast to rather simple treatment options including transfusion of platelets, plasma, and cryoprecipitate. The long turnaround time of laboratory coagulation testing, and intraoperative heparin use also make timely coagulation assessment difficult during cardiopulmonary bypass, and thus, hemostatic components are often empirically ordered and administered without knowing their actual need or efficacy. However, increasing clinical experience with viscoelastic coagulation testing in cardiac and transplant anesthesia has introduced a paradigm shift, enabling clinicians to obtain clinically relevant coagulation data in a timely fashion and to treat a specific element of coagulation that is dysfunctional. Viscoelastic coagulation testing may facilitate an optimal use of blood components and other hemostatic agents, but its application is often practice specific (ie, type of surgery), and there are technical limitations and learning curves. The aims of this review are thus to summarize recent clinical data on viscoelastic coagulation testing and to provide practical examples of its use in complex cardiac surgical and transplant cases.

[Laboratory coagulation tests in patients treated by direct oral anticoagulants (DOACs)]. / Bilan d'hémostase chez les patients traités par un anticoagulant oral direct (AOD).

Routine clotting time assays (Prothrombin Time/INR, activated Partial Thromboplastin Time [aPTT]) are prolonged at variable extent by direct oral anticoagulants (DOAC), according to the assay, the reagent and the type of DOAC. These assays are not reliable for monitoring the intensity of treatment and the measurement of the plasma level of the DOAC is usually not required. At high concentrations, DOAC interfere with the routine clotting assays, making them difficult to interpret. In critical situations such as major bleeding or urgent invasive procedure, the measurement of DOAC level and its kinetics are simple and useful to manage the patient.